Henry Bowditch in 1853 was the first to describe the occurrence of paraesophageal hernias. Based on post mortem findings he noted that “it (the esophagus) descended through the diaphragm as usual but turned back toward the left to enter the abnormal aperture caused by the hernia and to join the stomach in the chest”.

Eppinger, using a combination of auscultation and x-rays, is credited with making the diagnosis in a live patient. In 1919, Angelo Soresi published the first report on the elective surgical repair of a diaphragmatic hernia. In 1926, Ake Akerland, a Swedish radiologist, proposed the term hiatus hernia. A hiatal hernia is by definition the protrusion of abdominal viscera, most commonly the stomach and occasionally other organs, into the mediastinum through an enlarged esophageal hiatus of the diaphragm. Based on the relationship of the gastroesophageal junction to the herniated fundus of the stomach, and presence of other herniated abdominal viscera, hiatal hernias can be classified into 4 types.

In type 1 (sliding hiatal hernia) the gastroesophageal junction along with a variable portion of the proximal stomach migrates into the mediastinum, the stomach remains below the gastroesophageal junction, and thier longitudinal relationship is maintained. In type 2 (true paraesophageal hernia) the gastroesophageal junction remains in its normal position, and the fundus of the stomach herniates alongside the esophagus through disruptions in the phrenoesophageal membrane. In type 3 (combined paraesophageal hernia) the gastroesophageal junction herniates into the chest, as does the stomach with its fundus lying above the gastroesophageal junction.

In type 4 paraesophageal hernias there is the herniation of of the abdominal viscera into the mediastinum including the colon, small bowel, omentum, and spleen. A giant paraesophageal hernia is one in which at least 50% of the stomach is herniated into the chest, or on endoscopy the hernia has axial length of over 6 centimeters. Over 90% of hiatal hernias are type 1, paraesophageal hernias (type 2 to type 4) comprise about 5%. The remainder represent congenital (Morgagni hernia and Bochdalek Hernia) and traumatic hernias of the diaphragm. The etiology of paraesophageal hernias remains unknown, and is likely a combination of acquired and genetic factors. Familial clustering suggest a possible autosomal dominant mode. Additionally, mutations of a collagen-encoding gene, and altered collagen remodeling due to increased matrix metalloprotinase activity have all been suggested. The central acquired factor is chronically increased intra-abdominal pressure, as seen with central obesity, and with chronic constipation that results in disruption of the phrenoesophageal membrane with ultimate herniation.

The true prevalence of paraesophageal hernias remains unknown because a proportion remain asymptomatic, or are associated with non specific gastrointestinal complaints for which the association with the presence of a paraesophageal hernia is never made. When symptomatic, the type of symptoms vary with the size of the hernia. Small hernias, due to loss of barrier function of the lower esophageal sphincter from disruption of the phrenoesophageal membrane, loss of synergy between the high-pressure zone in the distal esophagus and the crural diaphragm, and loss of adequate intra-abdominal esophageal length, present most commonly with reflux related symptoms including heartburn and regurgitation. Medium-sized hernias often present with anemia. This results from linear erosions (Cameron Ulcers) in the gastric mucosa at the level of the diaphragmatic hiatus. Large paraesophageal hernias present with dyspnea,and chest pain or epigastric discomfort, all of which are more pronounced after eating. There is also early satiety due to distension of the herniated portion of the stomach with food with it is delayed passage into the infra diaphragmatic portion of the stomach.

On occasion, patient is present with an abdominal emergency due to acute gastric volvulus with Borchardt’s triad (severe epigastric pain, retching without the ability to vomit, and the inability to pass a nasogastric tube). Diagnostic workup involves multiple modalities. These include: Plain x-ray of the chest (retrocardiac air-fluid level, vertically oriented loops of bowel, upward displacement of the transverse colon, nasogastric tube coiled above the diaphragm), contrast esophagram (size and reduce ability of the hiatal hernia, relationship of gastroesophageal junction to hiatus, presence of a foreshortened esophagus, presence of esophageal diverticula), esophagogastroduodenoscopy (presence of esophagitis, biopsies for Barrett’s esophagus, gastric viability, size and type of paraesophageal hernia), computed tomography (gastric volvulus, intestinal obstruction, presence of additional organs besides stomach, location of gastroesophageal junction and type of paraesophageal hernia). pH testing to demonstrate abnormal proximal acid exposure (Demeester Score >14.7) identifies sliding type hiatal hernia is that would benefit from operative intervention, but is not critical in patients with paraesophageal hernias. Esophageal manometry is critical in identifying the type of anti reflux procedure that would be ideal for a given patient. Approach to the management of paraesophageal hernias is replete with controversy.

While there are proponents of watchful waiting in patients with asymptomatic or minimally symptomatic paraesophageal hernias (Stylopoulos N, 2002), the current consensus is to electively operate on patients who are acceptable candidate for surgery.This approach is supported by the fact that outcomes are significantly worse when emergent repair is needed. Similarly, there is general agreement that these hernias are best approached using minimally invasive laparoscopic techniques, and the operative approach is becoming progressively becoming more defined (DeMeester SR, 2013). Critical elements in repair of her paraesophageal hernia include a complete excision of the hernia sac, adequate mediastinal mobilization of the esophagus often up to the level of the inferior pulmonary ligament to gain approximately 3 centimeters of intra-abdominal esophagus, the use of a Collis gastroplasty when an adequate intra-abdominal length cannot be achieved, primary repair of the crura with relaxing incisions if needed to achieve a tension-free closure, reinforcement of the primary crural closure with a absorbable mesh, performance of a fundoplication to both prevent reflux as well as to place stomach between the mesh and the esophagus.

Yet, the holy grail in the laparoscopic repair of paraesophageal hernia is has yet to be achieved. One approach that holds promise is a hybrid approach that combines features of both the Nissen fundoplication, and the Hill repair. In this approach, in addition to performing a traditional Nissen fundoplication over an appropriately-sized bougie (56F in the majority, and 54F in smaller individuals), 2 additional sutures up placed between the anterior collar sling musculature, the posterior Collis sling musculature, and the pre aortic fascia. These additional sutures tethered the the fundoplication down in the abdomen and on long-term follow-up (mean follow-up 60 months) were associated with a anatomic recurrence rate of 5% versus 45% for patients undergoing a Nissen fundoplication alone (Shemmeri E 2018). The most controversial issue regarding the operative management of paraesophageal hernias relates to mesh reinforcement of the crura after crural plasty. Concerns for erosion of synthetic mesh led to an interest the use of biologic prosthesis as a means to reduce recurrence. In a prospective randomized multi center trial, Oeschlager et al. utilized porcine small intestinal submucosa to reinforce the crural repair in 108 patients.

At 6 months, there was a significantly lower rate of recurrence among the patients in whom a biologic prosthesis was used (9% versus 24% were no prosthesis was used, p=0.04). However, in the long-term these results did not hold out. Recurrence was identified in 54% of patients with biologic prosthesis reinforcement, and 59% in those with the primary repair alone (p=0.

7). The issue currently remains unresolved. Despite best efforts, a proportion of patient’s will recur. Radiologic recurrence must be distinguished from clinical recurrence. This is best achieved using a combination of clinical assessment, a contrast esophagram and pH testing. The majority of patients can undergo a redo fundoplication with hiatal hernia repair, performed laparoscopically.

A small proportion may require conversion to a Roux-en-Y esophago jejunostomy. In the multiply recurrent patients with disabling symptoms, esophagectomy may be needed as the last resort